Kumar Vijay, Sharma Bhavna, Nigam Abhay S
Department of Pathology, PGIMER, Dr. Ram Manohar Lohia Hospital, NEW DELHI, INDIA.
Turk Patoloji Derg. 2019;35(3):207-212. doi: 10.5146/tjpath.2018.01456.
Hemophagocytic Lymphohistiocytosis (HLH) is an uncommon, life-threatening hyperinflammatory syndrome, caused by severe hypercytokinemia, due to an overstimulated but ineffective immune process. The presenting features of HLH are non-specific, mimicking many other diseases, and hence its early recognition still remains a challenge. It requires a high index of suspicion and detailed analysis of clinical and laboratory findings to arrive at a conclusive diagnosis. The objective of this study is to present detailed clinical and laboratory features of a series of HLH cases.
A retrospective study was conducted wherein all the bone marrow aspirate smears diagnosed as HLH between January 2015 to June 2018 were reviewed. Detailed correlation of clinical and laboratory criteria was done with the bone marrow findings.
A total of twelve cases were diagnosed as HLH from January 2015 to June 2018. Ten patients fulfilled 5 out of 8 clinical and lab diagnostic criteria of HLH (2004). After correlating clinical and laboratory criteria along with bone marrow findings the diagnosis of HLH was suggested.
We present a series of twelve cases of Hemophagocytic Lymphohistiocytosis from a tertiary care hospital in New Delhi which will add not only to the understanding of this rare life threatening disease but also to the early diagnosis and intervention.
噬血细胞性淋巴组织细胞增生症（HLH）是一种罕见的、危及生命的高炎症综合征，由严重的高细胞因子血症引起，这是由于免疫过程过度刺激但无效所致。HLH的临床表现不具特异性，与许多其他疾病相似，因此其早期识别仍然是一项挑战。这需要高度的怀疑指数以及对临床和实验室检查结果进行详细分析才能得出确定性诊断。本研究的目的是呈现一系列HLH病例的详细临床和实验室特征。
进行了一项回顾性研究，对2015年1月至2018年6月期间所有诊断为HLH的骨髓穿刺涂片进行了复查。将临床和实验室标准与骨髓检查结果进行了详细的相关性分析。
2015年1月至2018年6月期间共诊断出12例HLH。10例患者符合HLH（2004年）8项临床和实验室诊断标准中的5项。在将临床和实验室标准与骨髓检查结果相关联后，提示了HLH的诊断。
我们展示了新德里一家三级护理医院的12例噬血细胞性淋巴组织细胞增生症病例系列，这不仅将增加对这种罕见的危及生命疾病的了解，还将有助于早期诊断和干预。
